Systemic treatment options to control tumor growth and symptoms related to hormone hypersecretion for patients with advanced or metastatic well-differentiated NETs arising in the gastrointestinal tract (gastrointestinal neuroendocrine tumors [GINETs], carcinoid tumors) will be discussed in this topic review. Systemic therapy options for pancreatic neuroendocrine tumors and for poorly-differentiated neuroendocrine carcinomas arising in the digestive tract are discussed elsewhere, as are local management options for well-differentiated metastatic gastroenteropancreatic NETs; the clinical presentation, imaging, and biochemical monitoring; pathology, and classification of gastroenteropancreatic NETs; clinical features of primary carcinoid tumors; diagnosis of carcinoid syndrome and tumor localization; the treatment of early-stage carcinoids; bronchial carcinoids; thymic carcinoids; management of symptoms of the carcinoid syndrome; and evaluation and management of neuroendocrine tumors of unknown primary site.

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